Spontaneous and recurrent pneumothorax revealing pulmonary Langerhans cell histiocytosis: clinical case report and literature review

Anne-Louise Kollegger(1), Vi Doan(2), Geneviève Derue(3) Published in the journal : January 2018 Category : Pneumology

Summary :

Langerhans cell histiocytosis is a rare disease that at times presents as an infiltrative lung disease, especially among smokers. We have herein reported the case of a young woman, who had been treated for histiocytosis in her childhood, presenting with spontaneous pneumothorax. CT scan revealed pulmonary Langerhans cell histiocytosis. A second pneumothorax episode with a permanent air leak made surgical treatment necessary. Given that the pneumothorax recurred, with the lung failing to expand, we conducted a literature review on this issue. This review revealed pneumothorax to occur in 10-20% of adult patients presenting pulmonary histiocytosis, with a high rate of recurrence.

What is already known about the topic?

Langerhans cell histiocytosis is rare, and its etiology is poorly understood. A pulmonary form may develop, especially in smokers, presenting as a pneumothorax.

What does this article bring up for us?

In recent years, significant progress has been made in the understanding of the disease, which suggests new therapeutic possibilities. Concerning the pneumothorax, which has a high risk of recurrence, recommendations are still missing, but the recent literature reveals surgical treatment to exhibit superior efficacy.

Key Words

Langerhans cell histiocytosis, pulmonary histiocytosis, pneumothorax.